Background/Purpose:

Little has been published on systemic sclerosis (SSc) patients requiring admission to Intensive Care Unit (ICU) for organ support; published results suggest respiratory failure due to end-stage lung disease the most frequent reason for admission and need for mechanical ventilation a significant predictor of poor outcome.

Methods:

Retrospective case note review of consecutive SSc patients admitted to ICU over a fifteen year period was analysed for patient demographic and disease characteristics, acute precipitating event, end organ damage requiring organ system support on ICU and outcome.

Results:

Fifty-one SSc patients were identified, 80%  female, 43% with diffuse cutaneous SSc and 41% with overlap syndromes; 24% (n=12) had clinically significant pulmonary fibrosis (PF), 12% (n=6) had pulmonary hypertension (PH), 14% (n=7) had cardiac SSc, 29% (n=15) had SSc renal crisis (SRC) and 12% (n=6) had severe SSc bowel involvement.

Sepsis (37%, n=19), pneumonia in particular (n=16), accounted for the single most frequent precipitant requiring ICU admission. Other primary reasons for admission included SRC (8%, n=4), cardiac (4%, n=2), neurological (6%, n=3) and non-infective pulmonary causes (6%, n=3). A large proportion of patients (29%, n=15) were on ICU post elective surgery.

All surgical, neurological or SRC admissions survived to ICU and hospital discharge. Sepsis on the other hand was associated with nearly 4 times higher risk of death within 1 year from ICU admission (HR 3.8, p=0.002) with 52% of these patients dying while on ICU compared to 13% of the other admissions (p=0.003).

Elective surgical patients spent up to 3 days on ICU, while the medical (n=33) and emergency surgical admissions (n=3) spent between 1 and 45 days (median 4, IQR 1, 19). On the day of admission 22% (n=8) required non-invasive mechanical ventilation, 61% (n=22) invasive mechanical ventilation, 22% (n=8) renal replacement therapy and 36% (n=13) vasopressors. Fourteen (39%) died while on ICU. While the use of mechanical ventilation and renal replacement therapy was not associated with negative short-term outcome, patients who needed vasopressor therapy were at nearly 6 times higher odds of dying while on ICU (OR 5.7, p=0.011).

Overall survival from ICU admission was 67% on day 7, 56% at 1 month and 44% at 1 year. Gender, subset, antibody specificities and overlap features did not associate with survival after ICU admission. On the other hand, compared to patients without significant organ involvement, those with PH had nearly 8 time increase of the risk of death (HR 7.7, p=0.024) and those with severe bowel disease had more than 5 times increase in the risk of death (HR 5.5, p=0.020). SRC on the other hand, was associated with good prognosis (HR 0.2, p=0.010).

Conclusion:

ICU outcomes of patients with underlying SSc seem to be better than previously reported, with best prognosis observed in post-surgical and SRC patients. Sepsis and pneumonia are most common medical precipitating events and are associated with poor prognosis. Mechanical ventilation and renal replacement therapy need is likely to reflect severity of underlying chronic disease and acute derangement and their use therefore should be guided by the overall clinical context.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/systemic-sclerosis-patients-on-intensive-care-unit-reasons-for-admission-and-determinants-of-outcome/